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IgG4-RD or immunoglobulin G4-related disease can affect almost any organ in the body. It’s manifestation is most often seen in the pancreas – autoimmune pancreatitis – or in the bile ducts and liver, where it is known as IgG4-related sclerosing cholangitis or IgG4-associated cholangitis.
The dramatic response to steroids makes autoimmune pancreatitis (AIP) unique among pancreatic disorders, but many cases are steroid-resistant, and relapse is common after steroid withdrawal. Mayo Clinic gastroenterologist, Dr. Mark Topazian discusses a recent study which shows that rituximab maintenance therapy can prolong remission.
So what are some "take home” messages from this study?
- Although patients with type 1 AIP have a dramatic response to steroid treatment, disease relapses are common.
- About 30 to 50 percent of the time, AIP returns, requiring additional treatment, sometimes long term.If relapse occurs after corticosteroid treatment or corticosteroid treatment is not tolerated, immunomodulators (IM) may be used. Immunosuppressants and immunomodulators include mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan).
- Nearly half of patients treated with IMs relapse during treatment.
- However, rituximab treatment is highly effective for induction and maintenance of remission in patients with autoimmune pancreatitis with or without IgG4-related sclerosing cholangitis, including those who are resistant to IM treatment.
Meet other people talking about autoimmune pancreatitis, including other IgG4-related diseases on Mayo Clinic Connect. Join the conversation, share experiences, ask questions, and discover your support network...
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Is there written documentation detailing the initial infusion dosage as well as the maintenance dosage and timeframe? I would like to see the complete study and recommendation.